A deeply personal post from Your Humble Blogger. This is something I wrote months ago about my own struggle with Muscular Dystrophy, but never intended to publish. I'm posting it now by request.
Q: How do you make God laugh?
A: Tell him your plans.
The young waitress paused at the doorway to the restaurant kitchen to casually talk to another staff member while effortlessly balancing a tray that must have been at least three feet in diameter. The tray was loaded with platters piled high with Tony Roma's barbeque - at least an entire tables order, yet it was being borne by the diminutive girl as though it was empty.
I watched in fascination while considering what such a tray must weigh. For me that act would be as impossible as levitation. In fact, I wouldn't be able to hold an empty tray in that manner due to a type of Muscular Dystrophy that has compromised my upper body muscles. From my perspective, this scene was akin to watching Chinese acrobats perform seemingly impossible feats of contortion. After the brief exchange the waitress moved off into the dining room without the slightest hint of stress or strain. Amazing.
Twenty four years ago I was a vigorous, active twenty something working full time in the local hospital, attending night school, and living for the weekends and days off when I could ride my bicycle into the nearby mountains or throw on a backpack disappear down a hiking trail. With strong legs and good endurance I'd always been about movement, and I was as at home in the outdoors as I was in my own living room.
One day word came down through the family grapevine that my cousin, then nineteen, had been diagnosed with Muscular Dystrophy. Like most people, my only image of M.D. then was of severely disabled kids in wheelchairs. And it was this vision I applied to my cousin as I cursed the fates that such an undeserving guy would suffer so. He was just beginning his life and now he was being told he'd most likely not even be able to feed himself at thirty. (A prediction that, thankfully, was entirely erroneous.)
Some months later I got a call one day from my Mom informing me that since our large family represented a good genetic sample, and researchers from the Duke University Medical Center Department of Neurology would like for all of the cousins, nineteen of us in all, to be tested for the disease. It was all just routine, you see. They simply wanted a family baseline. So, she asked, would I mind making an appointment with a local neurologist, get the evaluation, get some blood work, and let the physician forward the results on to Duke. The Muscular Dystrophy Association would even pay for it all, and it would be an enormous help to my cousin.
Naturally I was glad to help in way, and soon found myself in the doctor's office. It was an unusual exam. He had me sit down on the floor and get up, make a face, try to keep my eyes closed as the attempted to pry them open, resist downward and upward force with my arms, legs, etc. Nothing to it, really. So I was utterly astounded when he casually said, as he continued to write on my chart, that he was diagnosing me with the disease.
What? Wait, I'm only here for my cousin, I thought. Surely I misunderstood him. But there was no misunderstanding. He was telling me that I had Muscular Dystrophy. Never before or since have I been caught that "off guard." This doctor was a lot less pessimistic than the one who'd diagnosed my cousin. When asked what I could expect he explained that my upper body muscles would be affected and that I could expect fairly slow progression. I'd find some activities more difficult as the disease progressed. For example, getting a box down from an upper shelf would be difficult. (That prediction was dead on the money!) If anything, he painted a rosier picture than I'd ultimately realize.
I left his office in somewhat of a daze with a million questions begging to be answered and as many dire scenarios playing out in my mind. Still, I felt fine. Great, even. How could this be correct. Just the weekend before my buddy and I had been backpacking. But, as I continued to reflect a few things I'd experienced began to make sense. I'd never been able to develop much in the way of upper body strength. I'd lift weights and do pull ups, but my relatively weak upper body musculature had never matched my strong legs and robust cardiovascular health. I'd always written this off to lack of ardor on my part and pledged to do work harder at the gym. I recalled an incident when I was part of a crew painting a school and found that it very difficult to lift the brush higher shoulder level. I even caught a little razzing for that one. The evidence, these incidents and many others like them, began to stack up. It was time for a little research.
Facioscapulohumeral M.D. is among the milder of the muscular dystrophies. It follows an autosomal dominate genetic pattern meaning it's not linked to gender and that one parent must have that disease to pass it on. As it's name suggests it primarily affects the scapular, upper arm, and facial muscles, though other muscle groups are affected as well. And it's very unlike Duchenne's M.D. - generally regarded as the worst variety that drastically affects the very young and typically results in death in the teens or early adulthood. Instead, FSH is generally slow progressing, and given it's predominance in the upper musculature leaves most with fairly normal function and doesn't generally attack ambulation in the early stages and for some individuals never at all. Most victims of the disease will be able to function more or less normally late into life. Of course, as with any genetic trait the severity with which it's expressed will vary widely between individuals. So, some, like my Mom, might have zero expression, while extreme cases sometimes see patients 100% disabled in their twenties -
One classic trait of FSH is scapular winging. That's the tendency of the shoulder blades to, in effect, become un-moored and "wing" - or stick out - when the arms are extended. This looks rather like a chicken wing - hence the name. Since the scapula are no longer anchored properly, they no longer serve their proper role. So even while some arm and other muscles might still be fully functioning, the shoulder weakness will render even a modest weight unbearable.
Another effect is Lordosis. Weakening back and abdominal muscles will cause a lower spine curvature that results in an odd posture that make the patient appear to be leaning backwards. In time, this will affect ambulation.
Then there's facial weakness. There's actually a characteristic, mask-like look that some FSH victims have. Sort of an odd purse of the lips and a lack of expression. In fact the inability to whistle is one diagnostic indication.
So, it was obvious to me from my reading that FSH, while unfortunate, is actually quite mild in comparison to many of the other neuro-muscular disorders. It wouldn't shorten my life, it would most likely move slowly, and I'd live more or less normally for much of my life.
And, as I said before, I felt great. So I went on with my life as before, but with the looming threat of disability always in the background. I did woodworking, mountain biking, hiking, and all manner of other active stuff without much problem. My thirties came and with them a wife and two children. The yard was mowed, furniture built, fish caught, trails hiked, vacations enjoyed - in short I was able to take care of business despite the slowly progressive, virtually unnoticeable, weakening.
Able, that is, until the past five years or so. And now it seems that the effects of the disease have finally kicked into high gear. Sometime in that five year time frame I reached a tipping point that has seriously affected nearly every aspect of my life.
Where I was able to refinish some hardwood floors just a couple of years ago, I now am exhausted merely by detailing the interior of a car. Using a weed eater in the yard is out of the question. Ditto for pushing a lawn mower or working in my wood shop for any length of time. I could go on, but it's lets just say that the list of activities still within my ability is now drastically shorter than those without. I've now witnessed a steady parade of pleasurable activities march out of my life - each one dearly missed.
Most drastic of all is the hit to my ability to walk easily. My Lordosis, lower back curvature, is very pronounced. And renders me unable to stand up strait. I either lean backwards in n awkward fashion which causes my gait to be clumsy, or I stoop forward enabling me to walk a bit quicker, but brings about back pain. It also makes me look old. I now use a cane on a regular basis to help stabilize my gait. It's a little added security against falls which are a bit of a hazard.
For anything requiring more than a few brief minutes of walking - going to the mall, for example - I now have one of those electric scooters. How sexy is that?
That leaves me, at fifty, on edge about my future. At twenty I'd envisioned myself late in life as a fit, funky old guy on hiking up a mountain with a lifetime of adventures behind me and a happy trail before me. I was pretty much counting on my continued enjoyment of the outdoors to provide the satisfaction in my life as I blithely eschewed materialism. All I needed was a pair of boots and a guitar and I'd be fine as frog hair.
Now I fear that I could very well end up helpless and impoverished and a burden on those I love. An unthinkable and terrifying notion prior to diagnosis, but one that appears more realistic by the day.
I'm not sure if I'll be able to fully articulate this next bit. As I've become less able to handle simple tasks, I've had to rely on assistance more and more. This can be as simple as having to ask for help in moving a piece of furniture, but it diminishes you a little each time it happens. Imagine approaching something as simple a removing the oil cap from your engine and discovering that because of it's angle and location you can't generate the fairly modest amount of torque needed to get it done. Your engine needs a quart. It's a simple, every day procedure that shouldn't require even a seconds thought, but it's beyond your ability. For a man this is especially maddening. Strength is part and parcel to manliness, and to standby passively while others take up your slack is galling. Galling, too, is watching the weeds grow high, the paint peel, or the light bulb go unchanged. Men take care of business. It's what we do. But less so for me now, and this makes me less. Or, more precisely, makes me perceive myself as less. It's the threat that this disability might someday become total that keeps me awake at night.
That's the downside, here's the up: It could have been much worse. Though it's not pretty, I'm still walking and able to go to work every day. And while my uncertain future scares me, I'll be the first to admit that I've not had it that bad. I could have had something that would kill me young, or have spent my life in a wheelchair. As it is I've climbed mountains, loved women, swam rivers, danced (like a white guy, sadly), ran, played, built things, surmounted obstacles, fathered children, and enjoyed life. Far better people have had worse things happen to them. I'm happy to say that the same has been true for my cousin. He's a minister in North Carolina with two children. Like me he's still upright (physically and morally!), and living his life.
So my challenge now is to find a way to adjust my view of the world to accommodate what's happening to me. A new vision, I guess, of who and am what what my role is within my family and the world at large. I need to adopt an attitude of thankfulness for the richness of my past life. And I need to chart a course that will allow me to use my intact abilities to the fullest effect. As a wise man once said, "you have to go with your gifts."
(12/5/2010 Authors Update: I've found that way. More on that later...)